Safety and efficacy of noncardiac surgical procedures in the management of patients with trisomy 13: A single institution-based detailed clinical observation

Am J Med Genet A. 2018 May;176(5):1137-1144. doi: 10.1002/ajmg.a.38678.


Intensive treatment including surgery for patients with trisomy 13 (T13) remains controversial. This study aimed to evaluate the safety and efficacy of noncardiac surgical intervention for T13 patients. Medical records of patients with karyotypically confirmed T13 treated in the neonatal intensive care unit in Nagano Children's Hospital from January 2000 to October 2016 were retrospectively reviewed, and data from patients who underwent noncardiac surgery were analyzed. Of the 20 patients with T13, 15 (75%) underwent a total of 31 surgical procedures comprising 15 types, including tracheostomy in 10 patients and gastrostomy in 4. Operative time, anesthesia time, and amount of bleeding are described for the first time in a group of children with T13. All the procedures were completed safely with no anesthetic complications or surgery-related death. The overall rate of postoperative complications was 19.3%. Patients receiving tracheostomy had stable or improved respiratory condition. Six of them were discharged home and were alive at the time of this study. These results suggest at least short-term safety and efficacy of major noncardiac surgical procedures, and long-term efficacy of tracheostomy on survival or respiratory stabilization for home medical care of children with T13. Noncardiac surgical intervention is a reasonable choice for patients with T13.

Keywords: discharge; efficacy; noncardiac surgical intervention; safety; survival; trisomy 13.

MeSH terms

  • Disease Management
  • Female
  • Humans
  • Length of Stay
  • Male
  • Surgical Procedures, Operative* / adverse effects
  • Surgical Procedures, Operative* / methods
  • Treatment Outcome
  • Trisomy 13 Syndrome / diagnosis
  • Trisomy 13 Syndrome / surgery*